Short QT syndrome electrophysiologic studies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Among patients with SQTS, the atrial and ventricular refractory periods are shortened (ranging from 120 to 180 ms). Ventricular fibrillation can be induced on programmed stimulation in 90% of patients with short QT syndrome. Despite the high rate of VF inducibility, the risk of sudden death in an individual patient is difficult to predict given the genetic and clinical heterogeneity of short QT syndrome and the limited number of patients with short follow-up to date. The limitations of electrophysiologic testing are highlighted by a study of Giustetto et al in which the sensitivity of electrophysiologic testing in relation to the clinical occurrence of ventricular fibrillation was only 50% (3 of 6 cases)[1]. Importantly, lack of inducibility does not exclude a future episode of ventricular fibrillation[2]. Thus, the role of electrophysiologic testing in risk stratification of the patient with SQTS is not clear at present.

References

  1. Antzelevitch C, Pollevick GD, Cordeiro JM, Casis O, Sanguinetti MC, Aizawa Y, Guerchicoff A, Pfeiffer R, Oliva A, Wollnik B, Gelber P, Bonaros EP, Burashnikov E, Wu Y, Sargent JD, Schickel S, Oberheiden R, Bhatia A, Hsu LF, Haïssaguerre M, Schimpf R, Borggrefe M, Wolpert C (2007). "Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death". Circulation. 115 (4): 442–9. doi:10.1161/CIRCULATIONAHA.106.668392. PMC 1952683. PMID 17224476. Retrieved 2012-09-03. Unknown parameter |month= ignored (help)
  2. Schimpf R, Bauersfeld U, Gaita F, Wolpert C (2005). "Short QT syndrome: successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis". Heart Rhythm : the Official Journal of the Heart Rhythm Society. 2 (4): 416–7. doi:10.1016/j.hrthm.2004.11.026. PMID 15851347. Retrieved 2012-09-03. Unknown parameter |month= ignored (help)